副肿瘤性与特发性舞蹈病临床资料的对比
成人发作的舞蹈病通常会考虑Huntington，从而常常忽略了自身免疫型舞蹈病的诊断。Mayo临床中心的Orna O’Toole等学者描述了成人发作型自身免疫性舞蹈病的临床特点，并对比副肿瘤性与特发性两种亚型，其结果刊登在2013年3月19的Neurology杂志上。
该研究回顾性分析了Mayo临床中心自1997年至2012年15年内的36例自身免疫性舞蹈病患者的临床病例及实验室检查的数据，并对比22例非副肿瘤性及14例副肿瘤性舞蹈症。
该研究中女性患者21例，占58%。症状出现的评价年龄为67岁（范围为18-57岁）。估计Olmsted县的发病率为每百万患者年1.5。所有患者均为亚急性起病。20例为局部舞蹈症状，16例为全身舞蹈症状。尽管以舞蹈症状为主，但29例患者仍伴随出现其他神经症状，最常见的为眼睛运动异常（4例患者出现）。所有患者的抗NMDA受体抗体阴性，局限于基底节的免疫球蛋白IgG免疫荧光检测阴性。2例患者检测到与舞蹈症相关的突触IgG抗体(GAD65, 1和CASPR2, 1)。在副肿瘤性舞蹈症组，14例患者有明确的肿瘤史。其中13例为组织病理学证实的肿瘤，小细胞癌及腺癌最常见；6例患者检测到癌症预测性副肿瘤自身抗体，其中CRMP-5–IgG和ANNA-1是最多的。在特发性舞蹈症组，22例患者中19例有自身免疫方面的异常（最多为系统性红斑狼疮和抗磷脂抗体综合症），21例患者检测到自身抗体，出现最多的为狼疮及磷脂特异型（共19例患者）。副肿瘤组的年龄较高（p = 0.001），多数为男性(p = 0.006)，常见体重下降(p = 0.02)，并且多伴有周围神经病变(p = 0.008)。
研究结果发现，自身免疫型舞蹈病是一种相对罕见的疾病，进展较快。男性患者、年龄较高的、有严重舞蹈症状的、伴随周围神经病变及体重下降的患者更倾向于副肿瘤性舞蹈病。
OBJECTIVES:
To determine the characteristics of adult-onset autoimmune chorea, and compare paraneoplastic and idiopathic subgroups.
METHODS:
Thirty-six adults with autoimmune chorea were identified at Mayo Clinic (Rochester, MN) from 1997 to 2012. Medical record and laboratory data were recorded. Nonparaneoplastic (n = 22) and paraneoplastic cases (n = 14) were compared.
RESULTS:
Women accounted for 21 patients (58%). Median age at symptom onset was 67 years (range 18-87 years). We estimated the incidence for Olmsted County was 1.5 per million person-years. Symptom onset was subacute in all. Chorea was focal (20 patients) or generalized (16 patients). Although chorea predominated, other neurologic disorders frequently coexisted (29 patients); abnormal eye movements were uncommon (4 patients). No patient had NMDA receptor antibody or any immunoglobulin (Ig)G yielding a detectable immunofluorescence binding pattern restricted to basal ganglia. Two had synaptic IgG antibodies novel to the context of chorea (GAD65, 1; CASPR2, 1). In the paraneoplastic group, 14 patients had evidence of cancer. Of 13 with a histopathologically confirmed neoplasm, small-cell carcinoma and adenocarcinoma were most common; 6 patients had a cancer-predictive paraneoplastic autoantibody, with CRMP-5-IgG and ANNA-1 being most common. In the idiopathic group, 19 of the 22 patients had a coexisting autoimmune disorder (most frequently systemic lupus erythematosus and antiphospholipid syndrome); autoantibodies were detected in 21 patients, most frequently lupus and phospholipid specificities (19 patients). The paraneoplastic group was older (p = 0.001), more frequently male (p = 0.006), had more frequent weight loss (p = 0.02), and frequently had peripheral neuropathy (p = 0.008).
CONCLUSIONS:
Autoimmune chorea is a rare disorder with rapid onset. Male sex, older age, severe chorea, coexisting peripheral neuropathy, and weight loss increase the likelihood of cancer.
来源：丁香园
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